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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 190-191

Reversal of angiographic findings of moyamoya syndrome after congenital cyanotic heart disease repair

Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Date of Web Publication12-Jan-2017

Correspondence Address:
Dr. P N Sylaja
Comprehensive Stroke Care Program, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.198202

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How to cite this article:
Pande AC, Kesav P, Sreedharan SE, Sylaja P N. Reversal of angiographic findings of moyamoya syndrome after congenital cyanotic heart disease repair. Neurol India 2017;65:190-1

How to cite this URL:
Pande AC, Kesav P, Sreedharan SE, Sylaja P N. Reversal of angiographic findings of moyamoya syndrome after congenital cyanotic heart disease repair. Neurol India [serial online] 2017 [cited 2020 Aug 13];65:190-1. Available from:


Moyamoya disease is a chronic, occlusive cerebrovascular disease characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery (ICA) with abnormal vascular network at the base of the brain. It may be either idiopathic (moya moya disease; MMD) or can occur in conjunction with other conditions such as Down's syndrome, sickle cell disease (SCD), or congenital cyanotic heart disease (CCHD) [moya moya syndrome; MMS]. The natural history of MMS is variable with either occurrence of intermittent ischemic or hemorhagic events, or progressive cognitive decline. The association of MMS and congenital heart diseases (CHD) has been rarely reported, even though the causal role of the latter in the induction of MMS has not been proven. Reversible vascular changes in MMS are extremely uncommon, being reported with thyroid disease,[1] primary antiphospholipid syndrome,[2] and sickle cell disease (SCD)[3],[4] following effective treatment of the underlying etiology. However, these reversible vascular changes following a CCHD repair have not been reported as yet.

A 3-year-old female child, born of nonconsanguinous marriage with no antenatal or perinatal complications, was diagnosed to be having CCHD (tetralogy of Fallot) in the first postnatal week following evaluation for breathlessness. She was conservatively managed for the condition. At the age of 2 years, she had three episodes of transient ischemic attacks (TIA) involving the right and left hemispheres, lasting for 5–10 minutes each. Physical examination was unremarkable with no focal neurological deficits. Her brain magnetic resonance (MR) imaging showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities in the periventricular white matter in bilateral parieto-occipital and frontal region, with MR angiography showing stenosis of bilateral distal ICA, proximal anterior cerebral artery, and middle cerebral artery with extensive collaterals [Figure 1]a and [Figure 1]b suggestive of MMS. Workup for a secondary etiology was negative except for the finding of elevated hematocrit with a packed cell volume (PCV) of 53% (normal: 32–42%). Hence, a diagnosis of MMS secondary to polycythemia due to CCHD was made. A surgical revascularization procedure was planned for MMS on account of her symptomatic status; however, it was planed to be done only as a second step procedure after the correction of her underlying CCHD. She underwent an intracardiac repair of the CCHD with the development of postoperative left hemiparesis and secondary generalized seizures. Computed tomography (CT) of the head showed an acute infarct in the right frontal region and corona radiata. She underwent digital subtraction angiography of the cerebral vessels 3 months after the cardiac surgery prior to revascularization surgery for MMS, which, however, showed normalization of vessels with no moya moya collaterals [Figure 2]a and [Figure 2]b indicating spontaneous reversal of the angiographic findings of MMS following the CCHD repair. After surgery, the follow-up hematocrit also normalized with the PCV being 37%.
Figure 1: (a and b) Magnetic resonance angiography showing stenosis of bilateral distal internal carotid artery (Arrow), proximal anterior cerebral artery, and middle cerebral artery with basal collaterals (thick arrow)

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Figure 2: Four vessel digital subtraction angiography showing normal right internal carotid artery (ICA), middle cerebral artery, and bilateral anterior cerebral artery (a) and left ICA (b) [Left A1 is hypoplastic, left A1 territory is filling through anterior communicating artery, which is a normal variant]

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To the best of our knowledge, this is the first report of complete reversal of moya moya angiographic changes after intracardiac repair of an associated CCHD. This association may be either an incidental co-occurrence or could be accounted for by systemic factors such as hyperviscocity secondary to the CCHD leading to moya moya-like vascular changes. MMS has been associated with hematological disorders causing hyperviscosity such as sickle cell disease (SCD), essential thrombocythemia, and polycythemia vera. One previous report [5] hypothesized that sickled cells in SCD contributed to the occlusion of vasa-vasorum, leading to ischemia and progressive intimal, and medial vessel wall proliferation, thereby causing occlusion of the vessel lumen. In SCD, improvement in stenosis of cerebral vessels has been reported after treatment with chronic transfusion therapy, allogeneic bone marrow transplantation or hydroxyurea.[3],[4]

We hypothesize that hyperviscosity may have contributed to the cerebral vascular changes in our case as was evidenced by the normalization of angiographic changes in corroboration with the improvement in hyperviscosity after the CCHD surgery.

Our case emphasizes the importance of screening patients with CCHD and ischemic events associated with moya moya-like changes on angiography, with follow-up vascular imaging assessment after surgical treatment of CCHD, on account of the possible reversal of angiographic changes of MMS. Further research is required to identify the existence of any other factor causing stenosis of cerebral blood vessels in CCHD, which is reversed on intracardiac repair. This would provide an insight into the mechanisms causing intracranial vessel stenosis and MMS in those patients who have an underlying CCHD.

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There are no conflicts of interest.

  References Top

Ufuk U, Talip A, Yahya C, Dilek T. Reversible MR angiographic findings in a patient with autoimmune Grave's disease. AJNR Am J Neuroradiol 2004;25:1541-3.  Back to cited text no. 1
Booth F, Yanofsky BF, Ross IB, Lawrence P, Oen K. Primary antiphospholipid syndrome with moyamoya-like vascular changes. Pediatr Neurosurg 1999;31:45-8.  Back to cited text no. 2
Russell MO, Goldberg HI, Hodson A, Kim HC, Halus J, Reivich M, et al. Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood 1984;63:162-9.  Back to cited text no. 3
Steen RG, Helton KJ, Horwitz EM, Benaim E, Thompson S, Bowman L, et al. Improved cerebrovascular patency following therapy in patients with sickle cell disease: Initial results in 4 patients who received HLA-identical hematopoietic stem cell allografts. Ann Neurol 2001;49:222-9.  Back to cited text no. 4
Stockman JA, Nigro MA, Mishkin MM, Oski FA. Occlusion of large cerebral vessels in sickle-cell anemia. N Engl J Med 1972;287:846-9.  Back to cited text no. 5


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